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迟发性重症肌无力的胸腺切除术与抗肌肉自身抗体

Thymectomy and anti-muscle autoantibodies in late-onset myasthenia gravis.

作者信息

Romi F, Gilhus N E, Varhaug J E, Myking A, Skeie G O, Aarli J A

机构信息

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

出版信息

Eur J Neurol. 2002 Jan;9(1):55-61. doi: 10.1046/j.1468-1331.2002.00352.x.

Abstract

Thymectomy is still widely carried out in myasthenia gravis (MG) patients, but its role, especially in late-onset MG patients, is not established. These patients are immunologically heterogeneous, some with thymoma-like and others with early onset-like features. We evaluated whether any therapeutic effects of thymectomy correlate with the presence of non-acetylcholine receptor (AChR) muscle antibodies. The severity of MG, and titin and ryanodine receptor (RyR) antibodies, were assessed yearly starting from MG onset in 21 thymectomized and 22 non-thymectomized AChR antibody positive late-onset MG patients, who were followed for 2, 3 and 5 years. Clinical or pharmacological remission were seen in six of 11 titin antibody negative but none of the 10 titin antibody positive thymectomized patients, however, the non-thymectomized cases showed an opposite trend. The three MG-related deaths were all in patients with titin antibodies. There was no significant difference in MG severity between thymectomized and non-thymectomized patients; 2 years after MG onset, both groups were significantly improved. This study showed no dramatic benefit from thymectomy in late-onset MG in general. Any limited improvement appeared less likely in cases with titin and/or RyR antibodies.

摘要

胸腺切除术仍在重症肌无力(MG)患者中广泛开展,但其作用,尤其是在晚发型MG患者中的作用尚未明确。这些患者在免疫学上具有异质性,一些具有胸腺瘤样特征,另一些具有早发型样特征。我们评估了胸腺切除术的任何治疗效果是否与非乙酰胆碱受体(AChR)肌肉抗体的存在相关。从MG发病开始,每年对21例接受胸腺切除术和22例未接受胸腺切除术的AChR抗体阳性晚发型MG患者的MG严重程度、肌联蛋白和兰尼碱受体(RyR)抗体进行评估,随访2年、3年和5年。11例肌联蛋白抗体阴性的胸腺切除患者中有6例出现临床或药理学缓解,但10例肌联蛋白抗体阳性的胸腺切除患者均未出现缓解,然而,未接受胸腺切除术的患者呈现相反趋势。3例与MG相关的死亡均发生在有肌联蛋白抗体的患者中。胸腺切除患者和未接受胸腺切除术患者的MG严重程度无显著差异;MG发病2年后,两组均有显著改善。这项研究表明,总体而言,胸腺切除术对晚发型MG没有显著益处。在有肌联蛋白和/或RyR抗体的病例中,任何有限的改善似乎都不太可能出现。

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